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Vikram Paranjpe,1 Anat Galor,1,2 Pedro Monsalve,1 Sander R Dubovy,1 Carol L Karp11Department of Ophthalmology, University of Miami Miller School of Medicine, Miami, FL 33136, USA; 2Department of Ophthalmology, Miami Veterans Administration Medical Center, Miami, FL 33125, USAPurpose: This review will summarize the clinical and histological presentation of Salzmann nodular degeneration (SND), its prevalence and risk factors, potential underlying mechanisms, diagnostic tools, management options, and impact on cataract surgery and co-morbid ocular surface diseases.Method: PubMed review of 44 articles published between 1976 and 2018.Results: SND is a corneal disease characterized by whitish gray or bluish nodules on the peripheral or central cornea. The clinical presentation of SND is variable and the nodules can be asymptomatic or cause foreign body sensation and/or blurred vision. Histologically, SND appears as subepithelial nodules with thin overlying epithelium, disrupted or absent Bowman’s layer, and activated fibroblasts within the nodule. SND pathogenesis is not fully understood but is thought to involve poor epithelial protection and disruption of the epithelial–stromal interface, allowing for penetration of epithelially derived growth factors into the stroma and subsequent activation of stromal fibroblasts, eventually leading to sub-epithelial deposition of disorganized extracellular membrane components. SND most commonly occurs in Caucasian females in a bimodal distribution, occurring in the fifth or eighth and ninth decades of life. Risk factors for SND include ocular surface diseases and surgery. Surgical intervention is recommended in individuals with symptomatic nodules – primarily superficial keratectomy performed with or without intraoperative mitomycin C, photokeratectomy, and/or amniotic membrane transplantation. These procedures have been successful in removing the lesion and reducing corneal irregularity, but have variable recurrence rates (0–31%).Conclusion: The pathogenesis of SND is complex and multifactorial. Advances in diagnostic and treatment modalities have allowed for earlier and more accurate diagnosis and effective treatment of SND. Keywords: Salzmann nodular degeneration, cornea, diagnosis, imaging, treatment

Melina I Morkin,1,2 Rehan M Hussain,2 Ryan C Young,2 Tracy Ravin,2 Sander R Dubovy,2 Eduardo C Alfonso2 1Department of Ophthalmology, Shiley Eye Center, University of California – San Diego, San Diego, CA, 2Department of Ophthalmology, Bascom Palmer Eye Institute, Miller School of Medicine, University of Miami, Miami, FL, USA Abstract: A 51-year-old male who had undergone phacoemulsification in his left eye 11 months prior presented with complaint of sudden onset of blurred vision in the same eye. Review of his clinical course, slit-lamp exam, pachymetry, and specular endothelial microscopy led to the diagnosis of acute hydrops caused by Descemet’s membrane dehiscence at the site of the incision. He was initially managed with medical treatment and observation. In the subsequent months of follow-up, the corneal edema and the patient’s visual acuity did not improve. Intracameral gas injection was performed 7 months after presentation, but because of persistent corneal edema and nonattached Descemet’s membrane, penetrating keratoplasty was performed. Histopathologic examination confirmed the diagnosis. The patient has had a clear corneal graft since then. Although Descemet’s membrane detachment is a rather common complication after intraocular surgery, its unusually delayed presentation can also occur, and should not be confused with pseudophakic bullous keratopathy. Many mechanisms have been studied for the development of early tears and detachments after cataract surgery, but little is known about late presentations. The authors explore possible causes, and highlight the importance of instructing patients to avoid eye rubbing and any other type of trauma to the cornea after intraocular surgery. Keywords: Descemet’s membrane tear, detachment, cataract surgery, phacoemulsification

Veeral Shah, Leonid Zlotcavitch, Angela M Herro, Sander R Dubovy, Zohar Yehoshua, Byron L LamBascom Palmer Eye Institute, University of Miami, Miller School of Medicine, Miami, FL, USAAbstract: A 7-year-old girl presented with decreased vision in both eyes for 1 month. Examination showed visual acuity of 20/50 and 20/60, no afferent pupillary defect, cecocentral scotomas, and bilateral optic disc edema with extensive peripapillary and macular exudates. Magnetic resonance imaging showed multiple cortical and subcortical white matter lesions. Both the laboratory workup and the systemic examination were unrevealing. However, on follow-up, the patient showed episodic elevations of blood pressure as high as 240/160. Further workup revealed elevated urine catecholamines and a right supra-adrenal mass proven to be a pheochromocytoma by histopathologic analysis. The paroxysmal hypertension resolved, and the visual acuity, visual fields, fundus exam, and neuroimaging improved. The patient was lost to follow-up until age 18 when she developed shortness of breath and was found to have multiple pulmonary metastases identified as pheochromocytoma by biopsy. Genetic testing identified a 3p25-26 (c.482 G>A) VHL gene chromosomal mutation consistent with von Hippel–Lindau disease genotype. Multiple peripheral retinal vascular dilations and small retinal capillary hemangioblastomas were also found. This case highlights the importance of recognizing the lability of blood pressure often seen with pheochromocytomas, which may mask the underlying cause of hypertensive papillopathy and retinopathy, a diagnosis of low clinical suspicion in the pediatric population. The case also underscores the importance of thorough systemic workup, including genotyping to detect conditions where pheochromocytoma may be the presenting sign of the disease, such as multiple endocrine neoplasia 2A and 2B, von Hippel–Lindau disease, von Recklinghausen disease, tuberous sclerosis, and Sturge–Weber syndrome.Keywords: hypertensive encephalopathy, VHL, pheochromocytoma, paroxysmal hypertension

Luis J Haddock,1 Harry W Flynn Jr,1 Sander R Dubovy,1 Rahul N Khurana,2 Peter R Egbert31Department of Ophthalmology, Bascom Palmer Eye Institute, Miami Miller School of Medicine, Miami, FL, 2Northern California Retina Vitreous Associates, Mountain View, CA, 3Department of Ophthalmology, The Byers Eye Institute at Stanford, Stanford University, Palo Alto, CA, USAAbstract: A clinicopathologic correlation between two patients with acute-onset Aspergillus endophthalmitis undergoing enucleation is reported. These two patients presented with pain, redness, and decreased vision following uncomplicated cataract surgery. In both patients, vitreous aspiration and intravitreal injections were the initial treatment followed later by pars plana vitrectomy for clinical worsening. Despite repeated surgical and medical interventions, the clinical course of both patients was prolonged, unsuccessful, and resulted in enucleation for a blind painful eye. Histologic examination of the enucleated specimens showed that, in spite of prolonged local and systemic therapy, there was persistent diffuse infiltration of the anterior chamber and ciliary body by a filamentous mold.Keywords: mold, enucleation, voriconazole, amphotericin B

Hassan A Aziz1, Audina M Berrocal1,2, Robert A Sisk1, Kristin Hartley1, Magaly Diaz-Barbosa2, Rose A Johnson2, Ditte Hess1, Sander R Dubovy1, Timothy G Murray1, Harry W Flynn Jr11Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, 2Jackson Memorial Hospital, Miami, FL, USABackground: The purpose of this study was to report on the incidence and treatment outcomes of endogenous endophthalmitis among newborns in the neonatal intensive care unit (NICU) of a single medical center.Methods: This was a noncomparative, retrospective case series of endogenous endophthalmitis among infants at the Jackson Memorial Hospital NICU treated between March 1, 2002 and March 1, 2007.Results: Of 4323 infants admitted to the NICU, seven eyes of six (0.139%) infants (two males, four females) were diagnosed with endophthalmitis during the study period. Four patients were born prematurely with a mean gestational age of 27.5 weeks and a mean birth weight of 1153 g. Retinopathy of prematurity was reported in two of the six patients. Mean follow-up was 3.5 years. The diagnosis was confirmed by positive cultures or polymerase chain reaction testing at a median age of 34 postnatal days. Positive cultures included Candida albicans (n = 4), Pseudomonas aeruginosa (n = 1), and Herpes simplex type 2 (n = 1). All patients received systemic treatment and five received adjunctive ophthalmic interventions, including intravitreal antibiotics in five eyes of four patients and vitrectomy with pars plana lensectomy in three eyes. One patient underwent primary enucleation and another had delayed evisceration. In the remaining five eyes, there was a normal appearing posterior segment and normal intraocular pressures at last follow-up.Conclusion: Endogenous endophthalmitis is a rare complication in infants in the NICU, but may occur in patients with candidemia, bacteremia, retinopathy of prematurity, and low birth weight. Despite early and appropriate treatment, involved eyes may have poor outcomes.Keywords: endogenous endophthalmitis, neonatal intensive care unit

Localized orbital amyloidosis is a rare clinical entity. Periocular and orbital amyloid deposits are mainly located at the lacrimal apparatus, eyelid, conjunctiva, ocular adnexa, extraocular muscles, and levator palpebrae muscle. In this article, the authors report an unusual case of optic nerve amyloid deposition in an 82-year-old African American woman who presented with vertical diplopia. MRI revealed an enhancing mass from the optic nerve sheath, and CT showed foci of calcifications suggestive of optic nerve meningioma. However, an incisional biopsy demonstrated lymphoproliferative disease with focal optic nerve sheath amyloid deposition confirmed by histologic Congo red staining and immunohistochemistry., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: The aim of this study was to describe the clinical presentation, histopathologic characteristics, and management of phacolytic glaucoma., Patients and Methods: The database at the Florida Lions Ocular Pathology Laboratory was searched for surgical specimens at the Bascom Palmer Eye Institute. Patients with a diagnosis of "phacolytic glaucoma" on cytopathology between the years of 1997 and 2023 were included ( n  = 15). Patient demographics, anatomic site, laterality, clinical features, and ocular examination findings were obtained from available medical records., Results: The diagnosis of phacolytic glaucoma was established by light microscopic examination of cytology specimens in 15 eyes (Right = 8, Left = 6, not specified = 1). Between 1997 and 2023, there were 32 cytopathology cases with a preoperative diagnosis of phacolytic glaucoma. From this group of 15 cases, there were nine males and six females. Mean age was 64.2 years (Range: 39-87). Thirteen samples were from the anterior chamber and two were obtained from the vitreous. All 15 cases (100%) demonstrated histiocytes with engulfed foamy and/or granular presumed lenticular material. CD68 immunohistochemistry was positive within histiocytes in four cases that were stained (100%). Of the available medical records, clinical features, and ocular examination findings included: eye redness, decreased vision, eye pain, anterior chamber inflammation, dislocated or subluxed crystalline lens, cataract, and elevated intraocular pressure (average = 41.3 ± 8.67 mm Hg)., Conclusions: Phacolytic glaucoma is a rare complication of mature/hypermature cataracts that presents with ocular pain, decreased vision, and anterior chamber inflammation. AC paracentesis with cytopathologic evaluation is a minimally invasive, rapid technique that can aid in the diagnosis and management of this disease.

Adenoid cystic carcinoma (ACC) is a rare and lethal malignancy that originates in secretory glands of the head and neck. A prominent molecular feature of ACC is the overexpression of the proto-oncogene MYB. ACC has a poor long-term survival due to its high propensity for recurrence and protracted metastasis. Currently, clinical technologies lack the efficiency to distinguish patient prognosis prior to its redevelopment. We hypothesize that metastatic ACC can be detected by monitoring tumor-specific MYB expression in patients' blood. We developed a quantitative polymerase chain reaction (qPCR) assay for MYB transcripts and screened blood samples from four patient cohorts: no history or evidence of ACC (n=23), past history of ACC and no evidence of disease (NED) for greater than three years (n=15), local ACC (n=6), and metastatic ACC (n=5). Our assay detected significantly elevated levels of MYB transcripts in the metastatic ACC cohort (p < 0.01). Receiver operating characteristic (ROC) curves comparing metastatic to NED and metastatic to local disease were significant, with p values < 0.0001 and 0.0008, respectively. Single-cell RNA sequencing (scRNA-seq) of blood from metastatic ACC identified a cluster of circulating tumor cells (CTCs) expressing MYB. Here, we report a sensitive, cost-effective, and minimally invasive diagnostic test that leverages tumor-specific signatures to screen for metastatic ACC disease, potentially enhancing detection earlier than the current clinical standard., Competing Interests: Competing interests The authors declare that there are no competing interests.

Purpose: Ocular adnexal sebaceous carcinoma (OaSC) is an aggressive malignancy that often necessitates orbital exenteration. Its tumor composition and transcriptional profile remain largely unknown, which poses a significant barrier to medical advances. Here, we report the first in-depth transcriptomic analysis of OaSC at the single-cell resolution and discern mechanisms underlying cancer progression for the discovery of potential globe-sparing immunotherapies, targeted therapies, and biomarkers to guide clinical management., Design: Laboratory investigation with a retrospective observational case series., Methods: Single-cell RNA sequencing was performed on six patient specimens: three primary tumors, two tumors with pagetoid spread, and a normal tarsus sample. Cellular components were identified via gene signatures. Molecular pathways underlying tumorigenesis and pagetoid spread were discerned via gene ontology analysis of the differentially expressed genes between specimens. CALML5 immunohistochemistry was performed on an archival cohort of OaSC, squamous cell carcinoma, ocular surface squamous neoplasia (OSSN), and basal cell carcinoma cases., Results: Analysis of 29,219 cells from OaSC specimens revealed tumor, immune, and stromal cells. Tumor-infiltrating immune cells include a diversity of cell types, including exhausted T-cell populations. In primary OaSC tumors, mitotic nuclear division and oxidative phosphorylation pathways are upregulated, while lipid biosynthesis and metabolism pathways are downregulated. Epithelial tissue migration pathways are upregulated in tumor cells undergoing pagetoid spread. Single-cell RNA sequencing analyses also revealed that CALML5 is upregulated in OaSC tumor cells. Diffuse nuclear and cytoplasmic CALML5 staining was present in 28 of 28 (100%) OaSC cases. Diffuse nuclear and membranous CALML5 staining was present in 5 of 25 (20%) squamous cell carcinoma and OSSN cases, while diffuse nuclear staining was present in 1 of 12 (8%) basal cell carcinoma cases., Conclusions: This study reveals a complex OaSC tumor microenvironment and confirms that the CALML5 immunohistochemical stain is a sensitive diagnostic marker., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Purpose: Invasive fungal sinusitis (IFS) is associated with high rates of morbidity and mortality and often presents with orbital apex syndrome. Prompt diagnosis and management are crucial to prevent irreversible visual loss. We report a case of an immunosuppressed patient with rapidly progressive severe visual loss associated with frontal lobe cerebritis and leptomeningitis related to IFS, causing an adjacent compressive inflammatory optic neuropathy, which was treated successfully by large-dose corticosteroids., Observations: A 29-year-old woman with acute myeloid leukemia status post chemotherapy presented with right-sided headaches and periorbital swelling. Her examination was significant for subjective red desaturation and trace right eyelid edema and ptosis. The remainder of her initial ocular examination was normal. Her labs demonstrated neutropenia and thrombocytopenia. Imaging of the brain and orbits was concerning for extensive sinus disease with intracranial extension. An urgent multi-sinus and optic nerve decompression was performed given concern for compressive optic neuropathy, and the biopsy was consistent with invasive fungal infection. Despite aggressive antifungal treatment, vision in her right eye decreased rapidly to counting fingers. No optic nerve abnormalities were observed on serial MRIs, but adjacent inferior frontal lobe enhancement was present. After a vigorous debate in a multidisciplinary meeting, her severe vision loss was attributed to cerebritis causing an adjacent compressive inflammatory optic neuropathy, and large-dose intravenous (IV) steroid treatment was initiated while maintaining systemic antifungal therapy. Remarkably, she had a full recovery of her vision., Conclusions and Importance: Severe vision loss in IFS can occur due to compressive inflammatory optic neuropathy without direct fungal invasion as a contributing factor. Timely and effective intervention is crucial in preventing vision loss. Large-dose steroid therapy may be a potential treatment option for immunocompromised patients with invasive fungal sinusitis and intracranial invasion, provided strict fungal infection control measures are in place., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. The authors have no conflict of interest., (© 2024 The Authors. Published by Elsevier Inc.)

Background and Objective: This study evaluated the efficacy and durability of faricimab in patients with neovascular age-related macular degeneration (nAMD) who were previously treated with anti-vascular endothelial growth factor (anti-VEGF) agents., Patients and Methods: This retrospective case series was conducted at a single tertiary center in the United States. It focused on nAMD patients who transitioned to faricimab after initial anti-VEGF therapy, with a follow-up period of at least 9 months. "Complete dryness" was defined as the absence of intra- and/or subretinal fluid on optical coherence tomography. Durability was gauged by the extension of treatment intervals relative to the injection frequency of the previous agent., Results: Sixty-two eyes from 62 patients were included. Treatment interval ranged from 5 to 10 weeks; 10 (16%) patients were able to be extended by 2 or more weeks compared to their previous regimen. Median (interquartile range [IQR]) central field thickness was 310 μm (254, 376) on initiating faricimab and declined by the ninth month ( P values at 3, 6, and 9 months were 0.01, 0.02, and 0.07, respectively). Median (IQR) visual acuity at initiation of faricimab was 0.4 (0.20, 0.50) and did not change by the ninth month. Complete anatomical dryness was present in 10 (16%) eyes before switching; 90% remained dry at 9 months. Of 52 (84%) incompletely dry eyes before switching, 15% achieved complete dryness by 9 months on faricimab., Conclusions: Faricimab modestly improved the treatment intervals for a small proportion of previously treated patients on anti-VEGF therapy. [ Ophthalmic Surg Lasers Imaging Retina 2024;55:504-509.] .

Competing Interests: The authors have no financial or conflicts of interest to disclose.

Objective: To present a series of conjunctival keratoacanthomas and provide clinical, histopathological, immunohistochemical, and imaging results that characterize this rare entity., Methods: A retrospective chart review of records from 2005 to 2023 from the Florida Lions Ocular Pathology Laboratory was conducted. Nine patients with histologically proven conjunctival keratoacanthoma were identified. Data extracted includes demographics, clinical history, diagnostic testing, histopathological and immunohistochemical testing, treatment modalities, and recurrences., Results: Patients' mean age was 55.2 ± 21.1 years (range: 22-83). 77.8% (7/9) of patients were male. 55.6% (5/9) were Hispanic. 55.6% of lesions (5/9) were in right eyes. 55.6% of lesions (5/9) were on the temporal, bulbar conjunctiva. The lesions were rapidly growing, with mean onset time of 4.71 ± 3.30 weeks (range: 2-12). High-resolution anterior segment optical coherence tomography of three lesions revealed hyper-reflective, thickened epithelium with abrupt transition between normal and abnormal epithelium. Underlying disorganized subepithelial tissue was noted. However, the overlying abnormal epithelium caused considerable shadowing, which obscured subepithelial structures. Prominent, keratin-filled, cup-shaped lesions with faulty maturational sequencing that extend full thickness, variably pale cytoplasm, and foci of dyskeratosis and hyperkeratosis were present on all lesions' histopathology. All lesions were surgically excised, but two demonstrated partial spontaneous resolution before surgery. Two patients were lost to follow-up; the remaining seven had no signs of recurrence at a of mean of 36.9 ± 45.4 months (range: 3 to 141 months) of follow-up., Conclusions: Conjunctival keratoacanthomas are rare lesions of the ocular surface with distinct clinical, histopathologic, and diagnostic features., (Copyright © 2024 Canadian Ophthalmological Society. All rights reserved.)

Purpose: The purpose of this study was to describe the response of a papillomatous ocular surface squamous neoplasia (OSSN) to the intramuscular (IM) 9-valent human papillomavirus (HPV) vaccine after failed medical and surgical interventions., Methods: A 79-year-old White man with a conjunctival lesion underwent a biopsy which revealed OSSN and positivity for high-risk HPV. Initially treated with medical therapy and surgical excisions, the patient developed a recurrence and refused further surgery. He was given 4 doses of IM HPV vaccine at the 6-week interval., Results: A dramatic reduction in lesion size and reduced epithelial thickening and hyperreflectivity was noted on slitlamp examination and high-resolution anterior segment optical coherence tomography after receiving the IM HPV vaccine. Although lesion size was markedly reduced, the therapy did not achieve total resolution, resulting in further treatment with topical 1% 5-fluorouracil (5-FU) eye drops and later 0.04% mitomycin C eye drops. The patient then elected to discontinue further treatment and solely observe., Conclusions: This case report adds to the growing literature demonstrating the potential therapeutic use of vaccines in cancer treatment. Although HPV vaccination is currently approved for prophylaxis, the use of HPV vaccines as a therapeutic option for various HPV-mediated diseases, including OSSN, should be further explored. The HPV vaccine yielded significant initial improvement in this patient who refused further surgical interventions. The use of IM HPV vaccine as an adjunctive treatment of papillomatous OSSN may represent a potential therapeutic option in cases refractory to standard treatment modalities., Competing Interests: Drs. C. L. Karp and A. Galor have a pending PCT/US2022/029842 with the University of Miami. Dr. C. L. Karp is on the medical advisory board for Glaukos. Dr T. Ioannides holds patent 10 799 574 for the use of human papillomavirus vaccines for the treatment of malignant lesions. The remaining authors have no conflicts of interest to disclose., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Competing Interests: The authors report no conflicts of interest.

Purpose: Ocular dirofilariasis is an uncommon zoonotic infection that is usually associated with a carnivore host. In this case series and literature review, we investigate the clinical presentation, management, and histopathology of ocular dirofilariasis., Methods: The database at the Florida Lions Ocular Pathology Laboratory was searched for surgical specimens at the Bascom Palmer Eye Institute under approval of the Institutional Review Board. Patients with a histopathologic diagnosis of dirofilariasis between the years 1962 and 2022 from the Florida Lions Ocular Pathology Laboratory database were included ( n = 3). A systematic PubMed search was conducted by two independent authors to identify published cases of ophthalmic dirofilariasis worldwide. Keywords were used to identify articles, and exclusion criteria were applied., Results: Three patients, two males and one female, were identified from the Florida Lions Ocular Pathology Laboratory database with a diagnosis of ocular dirofilariasis. The mean age was 46.7 years (with a range 33-57 years). There were two eyelid lesions (Cases 1 and 3) and one involving the subconjunctival space (Case 2). All three organisms were excised and presumptively identified as Dirofilaria tenuis . All 3 patients were managed with curative surgical removal and recovered completely. Our review of the literature identified 540 published reports and 142 published reports with 186 cases that met the exclusion criteria., Conclusion: We present a case series and literature review of ocular dirofilariasis. Knowledge of the incidence, risk factors, prevention, and diagnosis of this unique parasitic infection will help in proper management and prevent further ocular complications., Competing Interests: The authors have no conflicts of interest to declare., (© 2023 S. Karger AG, Basel.)

Purpose: Lacrimal gland adenoid cystic carcinoma (LGACC) is a rare orbital malignancy with devastating lethality. Neoadjuvant intra-arterial chemotherapy (IACC) has demonstrated cytoreductive effects on LGACC macroscopically, but limited studies have examined cellular and molecular determinants of the cytoreductive effect. This post hoc study assessed apoptotic marker expression on excised tumor specimens after neoadjuvant IACC and globe-sparing resection, emphasizing the examination of tumor margins., Methods: This retrospective study identified LGACC specimens resected in a globe-sparing technique after neoadjuvant IACC by reviewing the Florida Lions Ocular Pathology database at Bascom Palmer Eye Institute. Histopathology slides of the specimens were re-examined to confirm the diagnosis and identify the tumor margin. Immunofluorescent staining was performed for apoptotic markers, including P53, cleaved caspase-3, cleaved PARP-1, and terminal deoxynucleotidyl transferase dUTP nick end labeling (TUNEL). Positive expression was determined by comparison to the negative control., Results: Tumor specimens from 5 patients met inclusion criteria. All 5 cases were positive at the center and the margin for TUNEL, p53, and cleaved caspase-3. One case did not show positive expression of cleaved PARP-1 at the margin but was positive for the other apoptotic markers., Conclusions: This post hoc study demonstrated positive staining for multiple apoptotic markers in post-IACC tumor specimens at the tumor center and margin. Apoptotic marker expression along the margins of post-treatment specimens is important, as it may offer surrogate information to speculate on the state of residual cancer cells adjacent to the excision margin inadvertently remaining in the orbit., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: Conjunctival melanoma is a rare neoplasm with high rates of recurrence and metastasis. Traditional management includes surgical excision and cryotherapy, followed by adjuvant therapy as needed. Immune checkpoint inhibitors, including nivolumab, are a targeted treatment option with improved survival rates. However, various immune-related adverse effects have been reported with these drugs. While systemic granulomatous inflammation is a documented systemic side effect, it has rarely been reported in the conjunctiva and ocular adnexa., Observation: A patient with a history of recurrent metastatic conjunctival melanoma presented with both a left sub-conjunctival and upper eyelid lesion after the commencement of treatment with nivolumab. The lesions were excised with a clinical suspicion for metastasis and consisted of noncaseating granulomatous inflammation with no evidence of malignancy on histopathologic examination. Infectious and primary autoimmune etiologies were ruled out., Conclusion and Importance: This is a biopsy-proven case of periocular immune checkpoint inhibitor-associated granulomatous inflammation., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 Published by Elsevier Inc.)

Purpose: This report describes the presentation of a 49-year-old woman with a branch retinal artery occlusion of the right eye in the setting of taking phentermine, a commonly used weight loss medication., Observations: A 49-year-old woman presented with acute painless vision loss in her right eye and was found to have a branch retinal artery occlusion after taking prescribed dosages of phentermine for weight loss therapy. Fundus examination revealed retinal whitening in the distribution of the superior temporal branch retinal artery, and spectral domain optical coherence tomography demonstrated macular edema. Systemic evaluation was negative for cardiovascular, infectious, or autoimmune etiologies. Based on the retinal findings, the patient was diagnosed with phentermine associated branch retinal artery occlusion. She was followed for nine years with no further complications and her vision remained stable in the right eye., Conclusions and Importance: This case highlights that phentermine, a commonly used weight loss medication, could be associated with ischemic retinopathies. Thus, clinicians should be aware that retinal vascular occlusions may not only occur in those who use recreational amphetamines but also in patients taking the prescribed dosages of a weight loss medication like phentermine., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Potential conflict of interest exists: We wish to draw the attention of the Editor to the following facts, which may be considered as potential conflicts of interest, and to significant financial contributions to this work: The nature of potential conflict of interest is described below: Philip J. Rosenfeld received research support from Carl Zeiss Meditec, Inc. He also received research funding from Gyroscope Therapeutics and Stealth BioTherapeutics. He is also a consultant for Apellis, Boehringer-Ingelheim, Carl Zeiss Meditec, Chengdu Kanghong Biotech, InflammX/Ocunexus Therapeutics, Ocudyne, Regeneron Pharmaceuticals, and Unity Biotechnology. He also has equity interest in Apellis, Valitor, and Ocudyne., (© 2024 Published by Elsevier Inc.)

A 93-year-old Caucasian woman presented to clinic for evaluation of a progressively enlarging left eyelid mass. Exam revealed a large, indurated, poorly mobile mass involving the left upper eyelid. Imaging demonstrated a crescentic enhancing soft-tissue mass in the left upper eyelid without evidence of deeper orbital involvement. Biopsy demonstrated tumor nests positive for cytokeratin AE1/AE3, P63, and P40 consistent with a diagnosis of eyelid lymphoepithelioma-like carcinoma (LELC). The patient underwent surgical resection with subsequent left upper eyelid reconstruction and adjuvant radiation. She had no clinical evidence of disease recurrence at follow-up. LELC is an uncommon tumor that rarely involves the eyelid and ocular adnexa. Early identification is important due to its malignant potential, as the majority of previously reported cases demonstrated local or distant metastatic spread. Future studies are needed to determine the optimum treatment regimen for this rare periorbital neoplasm.

An 89-year-old woman presented with chronic pain and foreign body sensation in a healthy-appearing anophthalmic socket. Computed tomography of the orbits showed hyperdense, cystic lesions superior and posterior to the orbital implant. Orbital exploration was performed; the orbital implant and lesions were removed. Histopathology revealed cystic structures composed of fibrocellular tissue lined with histiocytes and multinucleated giant cells, consistent with pseudocysts. Postoperatively, the patient noted the resolution of her symptoms. While the etiology of the pseudocysts remains unclear, we hypothesize that the answer can be traced back to the original surgery. The cysts may have formed after extravasation of fluid or proteinaceous material from the eye, from glycerin on the donor sclera, or after introduction of foreign material during retrobulbar injection of local anesthesia. This is the first report of pseudocysts occurring in the orbit posterior to an implant.

Purpose: The aim of this study was to describe a combined surgical approach for ocular surface and visual rehabilitation in patients with limbal stem cell deficiency, corneal scarring, and cataract. We aimed to introduce this combined approach as an alternative to a staged approach and to describe the intraoperative use of a diamond ophthalmic burr (DOB) and optical coherence tomography (OCT) to achieve and confirm a smooth graft-host interface during the anterior lamellar keratoplasty (ALK) portion of the procedure., Methods: The quintuple procedure described herein consists of a modified ALK, cataract extraction, intraocular lens implantation, simple limbal epithelial transplantation, and temporal tarsorrhaphy. Intraoperative OCT and DOB were used to guide the creation of the stromal bed during the ALK. The procedure was performed in a patient with limbal stem cell deficiency and corneal scarring after a chemical ocular burn., Results: The tarsorrhaphy was removed at 3 months postoperatively once complete corneal epithelialization was observed. The best-corrected visual acuity improved from light perception preoperatively to 20/30 at 16 months postoperatively with the use of scleral contact lenses. At this postoperative time point, the cornea was clear and compact, and the ocular surface was stable., Conclusions: The quintuple procedure allowed for visual and ocular surface rehabilitation in cases in which follow-up for a staged approach was not possible. Intraoperative OCT allowed for real-time visualization and dissection of the stromal bed, whereas the use of the DOB helped achieve a smooth stromal surface for an optimal graft-host interface., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Free-floating, pigmented vitreous cysts were documented in two patients. In a 15-year-old girl with intermittent symptoms, a 2.4-mm cyst was observed; origin was attributed to prior trauma, and clinical observation was pursued. In a 35-year-old woman with progressive symptoms, a 11.5-mm cyst was observed; origin was attributed to a history of multiple ocular surgical interventions, and surgical excision by pars plana vitrectomy was performed. [ Ophthalmic Surg Lasers Imaging Retina 2024;55:55-58.] .

Purpose: To describe the long-term management of bilateral limbal stem cell deficiency secondary to a severe chemical burn., Methods: Descriptive case report., Importance: This case highlights the importance of early intervention in ocular chemical burns for the preservation of tissue integrity and avoidance of perforation. We also review the use of proper ocular surface reconstructive techniques to restore the function of the limbal area, as well as the immunomodulatory strategies and follow-up needed for these interventions.

A 69-year-old woman with a history of a left orbital mass presented to the emergency room with progressive breakthrough pain in her left orbit despite medical therapy. On examination, there was extraocular motility restriction with diplopia upon left supraduction. Computed tomography (CT) scan of the orbits revealed soft tissue thickening of the left medial and superior periorbita and left lacrimal fossa; bony erosion of the left frontal bone, left orbital roof, and left lamina papyracea; and bilateral mass-like enlargement of the extraocular muscles. An orbitotomy with incisional biopsy was performed, and histopathological examination revealed non-caseating granulomatous inflammation consistent with sarcoidosis. Chest imaging demonstrated no sequela of pulmonary sarcoidosis, and her serum angiotensin converting enzyme (ACE) level was within normal range. She was treated with high-dose oral steroids with resolution of her symptoms. Her pain returned at the conclusion of the steroid taper, and it was controlled with chronic subcutaneous methotrexate and adalimumab injections.

Competing Interests: The authors have no financial or conflicts of interest to disclose.

Purpose: To characterize clinical and radiographic features, management, and outcomes of patients with orbital involvement of multiple myeloma (MM)., Methods: A retrospective chart review identified patients with MM and orbital involvement confirmed by histopathology at a single institution between 1995 and 2021. A comprehensive literature review was performed via PubMed to identify all previously reported cases of orbital MM., Results: Retrospective review identified 7 patients (43% male, mean age 68.7 years). Presenting symptoms included proptosis and diplopia. Orbital lesions were primarily located laterally (42.8%) with associated extraocular muscle (57.1%) or lacrimal gland (42.9%) involvement. Five patients (71.4%) had a previous diagnosis of systemic MM. Six patients received chemoradiation (85.7%). All patients had improvement of orbital disease with 2 patients deceased due to disease at follow-up (mean 8.9 months). Literature review identified 111 cases (46.8% male, mean age 58.6 years). 48.6% presented with orbital disease as the first manifestation of systemic MM. Lesions were most commonly located superolaterally (20.2%) with extraocular muscle infiltration (25.2%), lacrimal gland involvement (7.2%), and orbital bony destruction (39.6%). Treatments included chemoradiation, chemotherapy, or radiation alone. Approximately half (51.4%) of patients experienced improvement in orbital disease following treatment, and 48.6% were deceased at follow-up (mean 20.1 months)., Conclusions: This study provides a new retrospective study and updated comprehensive literature review regarding orbital MM. Given its poor prognosis, characterization of orbital MM is essential for early diagnosis. Orbital MM is often unilateral, located superolaterally, and may represent the first manifestation of systemic disease. Treatment includes chemotherapy and radiation, which may improve orbital disease; however, the overall prognosis remains poor., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Although primary tumors of the lacrimal gland are rare, adenoid cystic carcinoma (ACC) is the most common and lethal epithelial lacrimal gland malignancy. Traditional management of lacrimal gland adenoid cystic carcinoma (LGACC) involves the removal of the eye and surrounding socket contents, followed by chemoradiation. Even with this radical treatment, the 10-year survival rate for LGACC is 20% given the propensity for recurrence and metastasis. Due to the rarity of LGACC, its pathobiology is not well-understood, leading to difficulties in diagnosis, treatment, and effective management. Here, we integrate bulk RNA sequencing (RNA-seq) and spatial transcriptomics to identify a specific LGACC gene signature that can inform novel targeted therapies. Of the 3499 differentially expressed genes identified by bulk RNA-seq, the results of our spatial transcriptomic analysis reveal 15 upregulated and 12 downregulated genes that specifically arise from LGACC cells, whereas fibroblasts, reactive fibrotic tissue, and nervous and skeletal muscle account for the remaining bulk RNA-seq signature. In light of the analysis, we identified a transitional state cell or stem cell cluster. The results of the pathway analysis identified the upregulation of PI3K-Akt signaling, IL-17 signaling, and multiple other cancer pathways. This study provides insights into the molecular and cellular landscape of LGACC, which can inform new, targeted therapies to improve patient outcomes.

Carcinoma ex pleomorphic adenoma (CEPA) of the lacrimal gland is a rare malignant tumor that arises from a pre-existing pleomorphic adenoma. Lacrimal gland CEPA with mucoepidermoid histological subtype is exceedingly rare. Diagnosis can be aided by radiographic findings, though the gold standard is histopathological analysis following excisional biopsy. Management options include complete surgical excision with or without adjuvant radiation therapy based on tumor grade and invasiveness. We present a 76-year-old woman with 6 months of diplopia and unilateral proptosis. Her initial exam was remarkable for hypoglobus, proptosis, and limited elevation of the right eye. Computed tomography (CT) scan demonstrated a superior, well-circumscribed, extraconal orbital mass. An excisional biopsy was performed, and histopathological findings were consistent with mucoepidermoid carcinoma ex pleomorphic adenoma with positive margins in the tumor capsule. The patient received radiation therapy and remains markedly improved with no disease recurrence at 5 months post-operatively.

A 72-year-old woman with a history of chronic cocaine use presented 9 months after a dog bite with a large facial ulceration and absent sinonasal structures. Biopsies were negative for infectious, vasculitic, or neoplastic pathologies. The patient was lost to follow up for 15 months and returned with a significantly larger lesion despite abstinence from cocaine. Additional inflammatory and infectious workup was negative. Intravenous steroids were administered with clinical improvement. Therefore, she was diagnosed with pyoderma gangrenosum and cocaine-induced midline destructive lesion due to cocaine/levamisole. Pyoderma gangrenosum is a rare dermatologic condition that uncommonly involves the eye and ocular adnexa. Diagnosis involves clinical examination, response to steroids, exclusion of infectious or autoimmune conditions, and identifying potential triggers including cocaine/levamisole. This report highlights a rare presentation of periorbital pyoderma gangrenosum causing cicatricial ectropion associated with concomitant cocaine-induced midline destructive lesion and reviews important aspects of clinical manifestations, diagnosis, and management of pyoderma gangrenosum and cocaine/levamisole autoimmune phenomenon., Competing Interests: Dr. Thaller reports royalties from Thieme and Springer book publishing companies. The other authors have no relevant financial conflicts of interest. The authors alone are responsible for the content and writing of the paper., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: To report the clinical findings, treatments, and outcomes in a series of patients with vitreous metastasis from cutaneous melanoma., Methods: This single-center, retrospective, interventional case series included patients with biopsy-confirmed vitreous metastasis from cutaneous melanoma diagnosed between 1997 and 2020. Standard 23- or 25-gauge pars plana vitrectomy was performed for diagnostic sampling. Sclerotomies were treated with double or triple freeze-thaw cryotherapy. Perioperative intravitreal injections of melphalan (32 µg/0.075 mL) were administered, when indicated. Visual acuity, intraocular pressure, and systemic and ocular treatment responses were reported., Results: Five eyes of five patients with unilateral vitreous metastasis from cutaneous melanoma were identified. The median age at diagnosis was 84 (range, 37-88) years. The median follow-up after ophthalmic diagnosis was 28 (8.5-36) months; one patient did not have a follow-up. The initial visual acuity ranged from 20/30 to hand motions. Baseline clinical findings included pigmented or non-pigmented cellular infiltration of the vitreous (5/5), anterior segment (4/5), and retina (3/5). Four patients had secondary glaucoma. Systemic therapy included checkpoint inhibitor immunotherapy (n=3, all with partial/complete response), systemic chemotherapy (n=2), surgical resection (n=3), and radiation (n=2). The median time from primary diagnosis to vitreous metastasis was 2 (2-15) years. One patient had an active systemic disease at the time of vitreous metastasis. The final visual acuity ranged from 20/40 to no light perception. Ophthalmic treatment included vitrectomy in all five patients, intravitreal administration of melphalan in three, and intravitreal administration of methotrexate in one. One patient required enucleation, and histopathology revealed extensive invasion by melanoma cells., Conclusions: Vitreous metastasis from cutaneous melanoma can present as a diffuse infiltration of pigmented or non-pigmented cells into the vitreous and may be misdiagnosed as uveitis. Diagnostic pars plana vitrectomy and periodic intravitreal chemotherapy may be indicated.

Purpose: To examine the clinical and histopathologic characteristics of lacrimal gland biopsies at a tertiary academic center., Methods: A retrospective chart review of patients undergoing lacrimal gland biopsy or excision between 1962 and 2017 was performed via the ocular pathology specimen log. All cases were reviewed for demographics, clinical presentation, and histopathologic diagnoses., Results: Four hundred and two eyes in 356 patients were included in the analysis. Median age was 49 (range 5-91) with a female predominance (255, 72%, p < .001). Most had unilateral involvement (308, 86.5%) and visual acuity of 20/50 or better (332 eyes, 83%). Limitation in extraocular motility was present in 71 eyes (18%), relative afferent pupillary defect in 10 eyes (2.5%), and intraocular pressure 20 mmHg or above in 80 eyes (20%). The pre-operative radiology report commented on the enlargement of the lacrimal gland in 236 eyes (58.7%), and lack thereof in 73 eyes (18.2%). The most common histopathologic diagnoses were nonspecific inflammation or orbital pseudotumor (170, 42%), lymphoma (65, 16%), pleomorphic adenoma (22, 5.5%), adenoid cystic carcinoma (19, 4.7%), granulomatous inflammation (19, 4.7%), and normal lacrimal gland (16, 4%). Three hundred and seven cases were benign (76%) and 95 malignant (24%). The biopsy specimen was diagnostic in 343 (85%), and non-diagnostic in 59 (15%)., Conclusions: This is a comprehensive review of one of the largest ocular pathology databases of lacrimal gland lesions. This study confirms the wide range of inflammatory and neoplastic conditions affecting the lacrimal gland and highlights the nuances of histopathologic diagnoses and diagnostic yield of biopsies in clinical practice.

Purpose: To describe the anterior segment optical coherence tomography (AS-OCT) appearance of conjunctival papilloma and identify differentiating features from papilliform ocular surface squamous neoplasia (OSSN)., Method: A retrospective chart review of individuals clinically diagnosed with conjunctival papilloma (n = 10) or papilliform OSSN (n = 10) based on slit lamp features. Data on demographics, tumour characteristics, and primary treatment were collected. AS-OCT features were assessed including epithelial thickness and reflectivity, a corrugated epithelial surface, presence of an overhanging edge, presence of intrinsic spaces and posterior shadowing. Histopathology was available in 5 papilloma and 3 OSSN specimens., Result: Overall, the majority of individuals in both groups were white males. OSSN lesions were more likely to involve the limbus (80% vs.10%, p = 0.005) and the bulbar conjunctiva (100% vs. 20%, p < 0.001) compared to papillomas. On AS-OCT, maximum epithelial thickness was thicker in papilloma compared to OSSN (936 ± 533 vs. 637 ± 207 µm, p = 0.009). The feature that best differentiated papilloma from OSSN was an overhanging edge (100% vs. 0%, p < 0.001), where the epithelial lesion was seen on top of underlying normal epithelium. Other features more common in papilloma compared to OSSN included a corrugated epithelial surface (70% vs.10%, p = 0.02), the presence of intrinsic spaces (100% vs. 50%, p = 0.03), and posterior shadowing (100% vs. 40%, p = 0.01)., Conclusion: AS-OCT shows differentiating features between papilloma and OSSN with an overhanging edge as a distinctive AS-OCT feature of papilloma., (© 2022. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

Myxofibrosarcoma (MFS), a common sarcoma in adults, typically affects the extremities. Primary orbital involvement is uncommon, and orbital metastasis is exceedingly rare. We describe a case of orbital MFS due to metastasis and provide an updated literature review. A 56-year-old woman presented with 2 months of progressive right eye proptosis. Relevant history included left palmar MFS excised 2 years prior. Examination demonstrated proptosis and restriction in abduction of her right eye without optic nerve compromise. Imaging revealed a well-circumscribed right orbital mass. Initial biopsy showed glandular tissue with chronic inflammation without an evidence of malignancy. She subsequently developed worsening proptosis, complete ophthalmoplegia, and a new afferent pupillary defect. Repeat orbital excisional biopsy with deeper dissection demonstrated high-grade MFS consistent with metastasis. She underwent chemoradiation with resolution of her lesion. Literature review identified 12 previously reported cases of MFS, only two of which involved metastasis to the orbit and were associated with worse prognosis. This report highlights a rare case of orbital metastasis of MFS with high-grade features confirmed on histopathology and provides an updated literature review. Prompt diagnosis and complete removal of MFS located both systemically and in the orbit are essential due to the metastatic potential., Competing Interests: The authors declare that there are no conflicts of interests of this paper., (Copyright: © 2023 Taiwan J Ophthalmol.)

A 34-year-old man presented with recurrent bilateral periorbital swelling and pain for 16 years after receiving facial fillers of an unknown substance in a hotel room from a stranger claiming to work in a medical office. Exam demonstrated a firm, mildly tender nodule along the right upper cheek. Imaging revealed a tubular hyperdensity in the right premaxillary soft tissues. Lower eyelid and upper cheek dissection resulted in retrieval of a tubular metallic foreign body consistent with a needle. Histopathology of surrounding tissue demonstrated iron deposition with granulomatous inflammation. Periocular fillers are a common aesthetic procedure. Although generally well-tolerated, complications include inflammatory reactions, infection, necrosis, and vision loss. This case highlights retention of a metallic foreign body, a complication of filler injection that has not been previously reported, emphasizing the importance of careful injection technique by licensed professionals and imaging and surgical exploration if a foreign body is suspected., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2022 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

A 33-year-old pregnant woman presented with six months of right-sided proptosis. Neuroimaging revealed a right orbital arteriovenous malformation arising from the second segment of the ophthalmic artery. As she was 9 weeks pregnant, the decision was made to monitor her closely. Over the following six months, her proptosis progressed, accompanied by decreased visual acuity, afferent pupillary defect, and red desaturation concerning for compressive optic neuropathy. After planned c-section, she underwent embolization with n-butyl cyanoacrylate. Upon awakening after embolization, she had no light perception vision from her right eye and was found to have ophthalmic artery obstruction. She ultimately developed a blind painful right eye and underwent enucleation with histopathology demonstrating glue in the central retinal artery, posterior ciliary arteries, and choroid. This case highlights ophthalmic artery occlusion as a rare complication of orbital arteriovenous malformation embolization and demonstrates correlating histopathological findings, which have not previously been reported.

Purpose: The purpose of this study was to report the first case of a conjunctival granulomatous lesion as the presenting sign of granulomatous polyangiitis (GPA) in a pediatric patient., Methods: This study is a case report., Results: A 14-year-old Hispanic boy presented with a conjunctival lesion on the inferior bulbar conjunctiva of the right eye associated with diffuse conjunctival injection. The mass progressively grew and became painful over the course of 6 weeks. No retinal or orbital abnormalities were noted on examination. The lesion was excised, and histopathological analysis was consistent with granulomatous inflammation. The lesion recurred after 15 months, and a second excisional biopsy was performed. The lesion again slowly recurred, and on presentation to our clinic, an elevated lesion in the inferior limbal/bulbar conjunctiva of the right eye was noted from 4 to 8 o'clock with accompanying forniceal shortening. Five months after the second excision, the patient developed flu-like symptoms with polyarthralgia. A full diagnostic workup revealed multiple pulmonary nodules on chest imaging, proteinuria on urinalysis, and a positive c-antineutrophil cytoplasmic antibody on serological studies. Based on these findings, the patient underwent a kidney biopsy which showed pauci-immune crescentic glomerulonephritis, consistent with a diagnosis of GPA. The patient achieved disease remission with rituximab. Despite treatment, the conjunctival lesion did not regress and remained unchanged in size for 3 years with periodic episodes of inflammation., Conclusions: This is the first documented case of a conjunctival mass as the initial presenting feature of pediatric GPA. The presence of granulomatous inflammation on histopathology and recurrences after excision should raise suspicion for GPA in children and adults., Competing Interests: The authors have no funding or conflicts of interest to disclose. The authors have no funding or conflicts of interest to disclose., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Orbital sarcoid is a rare entity and may be the first manifestation of systemic sarcoidosis. We report a case of orbital sarcoidosis where diagnosis was complicated by a history of lower eyelid blepharoplasty. The patient presented with progressive swelling of the left lower eyelid, which was assumed to be a late complication of her surgery. After failing multiple treatments, MRI orbits was obtained and revealed an enhancing lesion in the left orbit inseparable from the lacrimal gland and inferior oblique muscle. Biopsy showed noncaseating granulomatous inflammation, and the patient was eventually diagnosed with sarcoidosis., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2022 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose of Review: The aim of this study was to review the existing literature and investigate the role of microbiologic culture and histopathologic examination of corneal biopsies in the management of infectious keratitis., Recent Findings: Corneal biopsy continues to be a significantly useful tool in the diagnosis and tailored management of infectious keratitis. Several techniques can be employed for tissue collection, handling and processing to optimize diagnostic yield and maximize safety, including emerging femtosecond laser-assisted biopsy., Summary: Corneal opacities represent a significant cause of global blindness, and infectious keratitis is the most common cause. Organism identification in progressive infectious keratitis is essential for proper management. However, microbiological culture alone has a high rate of false-negative results. Records from the Bascom Palmer Eye Institute were retrospectively searched for patients between 1 January 2015, and 31 December 2019, who underwent corneal biopsy, therapeutic keratoplasty or endothelial graft removal for infectious keratitis and had specimens bisected and submitted for evaluation with both microbiologic culture and histopathologic examination. Detection of bacteria, fungus and mycobacteria was not statistically different between culture and histopathology. Microbiology and histopathology are complementary methods for the identification of causative microorganisms in corneal specimens with presumed infectious keratitis., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Squamoid eccrine ductal carcinoma (SEDC) is a rare cutaneous neoplasm that often manifests as a plaque or nodule in sun-exposed areas of older patients. Herein, the authors report the first case of SEDC in the eyelid. A 76-year-old man presented with a 2.5 × 1.5 mm area of left upper eyelid erythema, thickening, ulceration, and scaling with madarosis superotemporally just above the lash line. Full-thickness wedge biopsy demonstrated irregular epithelial tubules with nuclear atypia and focal squamous differentiation, consistent with SEDC. The patient underwent Mohs resection and has had no recurrence approximately 27 months after surgical removal. The authors present this case to raise awareness of SEDC to ophthalmologists as all previous cases have been described in the nonophthalmic literature. A full-thickness biopsy is recommended to avoid misdiagnosing SEDC as squamous cell carcinoma (SCC), a less aggressive tumor. With greater awareness, there may be increased recognition of this likely underreported, more malignant entity., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2022 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: The purpose of this study was to evaluate the risk of symptomatic severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection after corneal transplantation surgery, with cataract surgeries as controls, and the impact of the novel coronavirus disease pandemic in the clinical and surgical complications of corneal transplantation and cataract surgeries., Methods: A retrospective matched case-control study of 480 consecutive individuals who underwent surgery at the Bascom Palmer Eye Institute between May 2020 and November 2020. A total of 240 patients who underwent corneal transplantation with tissue obtained from the Florida Lions Eye Bank were age, race, ethnicity, and sex matched with 240 patients who underwent cataract surgery during the same day and by the same surgical team. Only the first corneal transplant or cataract surgery during this period was considered for each individual. All donors and recipients were deemed SARS-CoV-2 negative by a nasopharyngeal polymerase chain reaction test before surgery. Postoperative SARS-CoV-2 infections were defined as previously SARS-CoV-2(-) individuals who developed symptoms or had a positive SARS-CoV-2 polymerase chain reaction test during the first postoperative month., Results: Mean age, sex, race, and ethnicity were similar between groups. There were no differences between the corneal transplant and cataract groups in the rates of SARS-CoV-2 infection before (5.8% vs. 7.5%, P= 0.6) or after surgery (2.9% vs. 2.9%, P = 1). The rates of postoperative complications did not increase during the pandemic, compared with previously reported ranges., Conclusions: In this study, postoperative SARS-CoV-2 infection was similar for individuals undergoing corneal transplantation or cataract surgery. Further research is required to evaluate the transmission of SARS-CoV-2 through corneal tissue., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: Gain of chromosome 6p has been associated with poor ocular survival in retinoblastoma and histopathologic grading of anaplasia with increased risk of metastatic spread and death. This study examined the correlation between these factors and other chromosomal abnormalities as well as results of whole genome sequencing, digital morphometry, and progression-free survival., Design: Retrospective cohort study from 2 United States tertiary referral centers., Participants: Forty-two children who had undergone enucleation for retinoblastoma from January 2000 through December 2017., Methods: Status of chromosomes 6p, 1q, 9q, and 16q was evaluated with fluorescence in situ hybridization, the degree of anaplasia and presence of histologic high-risk features were assessed by ocular pathologists, digital morphometry was performed on scanned tumor slides, and whole genome sequencing was performed on a subset of tumors. Progression-free survival was defined as absence of distant or local metastases or tumor growth beyond the cut end of the optic nerve., Main Outcome Measures: Correlation between each of chromosomal abnormalities, anaplasia, morphometry and sequencing results, and survival., Results: Forty-one of 42 included patients underwent primary enucleation and 1 was treated first with intra-arterial chemotherapy. Seven tumors showed mild anaplasia, 19 showed moderate anaplasia, and 16 showed severe anaplasia. All tumors had gain of 1q, 18 tumors had gain of 6p, 6 tumors had gain of 9q, and 36 tumors had loss of 16q. Tumors with severe anaplasia were significantly more likely to harbor 6p gains than tumors with nonsevere anaplasia ( P < 0.001). Further, the hematoxylin staining intensity was significantly greater and that of eosin staining significantly lower in tumors with severe anaplasia ( P < 0.05). Neither severe anaplasia ( P  = 0.10) nor gain of 6p ( P  = 0.21) correlated with histologic high-risk features, and severe anaplasia did not correlate to RB1 , CREBBP , NSD1 , or BCOR mutations in a subset of 14 tumors ( P > 0.5). Patients with gain of 6p showed significantly shorter progression-free survival ( P  = 0.03, Wilcoxon test)., Conclusions: Gain of chromosome 6p emerges as a strong prognostic biomarker in retinoblastoma because it correlates with severe anaplasia, quantifiable changes in tumor cell staining characteristics, and extraocular spread., (© 2021 by the American Academy of Ophthalmology.)